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{{Cyanosis}}
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{{CMG}}; {{AE}}  
{{CMG}}; {{AE}} {{Sara.Zand}}  


==Overview==
==Overview==
Cardiac defects causing [[central cyanosis]] include: [[Transposition of the great arteries]], [[Tetralogy of fallot]], [[Tricuspid atresia]], [[Truncus arteriosus]],[[Total anomalous pulmonary venous connection]], [[Ebstein anomaly]], critical [[Pulmonary stenosis]] or atresia, functional [[single ventricle]]. The palliative surgical shunt maybe done in such lesions to increase [[pulmonary blood flow]] even in the presence of [[cyanosis]]. Complete repair procedure leads to relief of cyanosis and shunt and also has long term complications.


== Cardiac catheterization ==
== Recommendation for surgery in [[cyanotic heart disease]] ==
* Cardiac catheter interventions can be palliative by improving cyanosis or be corrective by relieving obstruction to flow.
The table shows indications for surgery in [[cyanotic congenital heart disease]] according to 2018 [[AHA/ACC Guideline]]:<ref name="StoutDaniels2019">{{cite journal|last1=Stout|first1=Karen K.|last2=Daniels|first2=Curt J.|last3=Aboulhosn|first3=Jamil A.|last4=Bozkurt|first4=Biykem|last5=Broberg|first5=Craig S.|last6=Colman|first6=Jack M.|last7=Crumb|first7=Stephen R.|last8=Dearani|first8=Joseph A.|last9=Fuller|first9=Stephanie|last10=Gurvitz|first10=Michelle|last11=Khairy|first11=Paul|last12=Landzberg|first12=Michael J.|last13=Saidi|first13=Arwa|last14=Valente|first14=Anne Marie|last15=Van Hare|first15=George F.|title=2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines|journal=Circulation|volume=139|issue=14|year=2019|issn=0009-7322|doi=10.1161/CIR.0000000000000603}}</ref>
* Balloon valvuloplasty can be effective in patients with critical pulmonary stenosis or aortic stenosis. Selected patients with pulmonary atresia are also candidates for balloon valvuloplasty if the obstruction is membranous, the tricuspid annulus and right ventricular size are adequate to support a two ventricle repair, and the coronary circulation does not depend upon the right ventricle [10].
<ref name="ShiZhu2017">{{cite journal|last1=Shi|first1=Guocheng|last2=Zhu|first2=Zhongqun|last3=Chen|first3=Jimei|last4=Ou|first4=Yanqiu|last5=Hong|first5=Haifa|last6=Nie|first6=Zhiqiang|last7=Zhang|first7=Haibo|last8=Liu|first8=Xiaoqing|last9=Zheng|first9=Jinghao|last10=Sun|first10=Qi|last11=Liu|first11=Jinfen|last12=Chen|first12=Huiwen|last13=Zhuang|first13=Jian|title=Total Anomalous Pulmonary Venous Connection|journal=Circulation|volume=135|issue=1|year=2017|pages=48–58|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.116.023889}}</ref><ref>{{cite journal|doi=10.4103/2F0974-2069.157025}}</ref><ref name="Attenhofer JostConnolly2007">{{cite journal|last1=Attenhofer Jost|first1=Christine H.|last2=Connolly|first2=Heidi M.|last3=Dearani|first3=Joseph A.|last4=Edwards|first4=William D.|last5=Danielson|first5=Gordon K.|title=Ebstein’s Anomaly|journal=Circulation|volume=115|issue=2|year=2007|pages=277–285|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.106.619338}}</ref><ref name="pmid27275259">{{cite journal |vauthors=Idrizi S, Milev I, Zafirovska P, Tosheski G, Zimbakov Z, Ampova-Sokolov V, Angjuseva T, Mitrev Z |title=Interventional Treatment of Pulmonary Valve Stenosis: A Single Center Experience |journal=Open Access Maced J Med Sci |volume=3 |issue=3 |pages=408–12 |date=September 2015 |pmid=27275259 |pmc=4877828 |doi=10.3889/oamjms.2015.089 |url=}}</ref>


== Respiratory distress syndroem ==


=== Surfactant therapy ===
Exogenous surfactant replacement therapy is effective in reducing RDS mortality and morbidity in preterm infants [27-30]. Several clinical trials have shown the benefit of surfactant administration in preterm infants born less than 30 weeks gestation who are at the greatest risk for RDS [27,29,31,32]. In these trials, surfactant therapy compared with placebo was associated with a lower incidence and severity of RDS and mortality, and a decreased rate of associated complications including BPD, pulmonary interstitial emphysema, and other pulmonary leak complications, such as pneumothorax [27,29,31,32].


When surfactant therapy is used, the following issues must be addressed:
'''<span style="font-size:85%">'''Abbreviations:'''
'''d-TGA:''' [[dextro-Transposition of great arteries]];
'''PDA:''' [[Patent ductus arteriosus]]  ;
'''ASD:''' [[Atrial septal defect]];
'''VSD:''' [[Ventricular septal defect]];
'''TAPVC:''' [[Total anomalous pulmonary venous connection]];
'''TOF:''' [[Tetralogy of fallot]];
'''CCTGA:''' [[Congenitally corrected transposition of the great arteries]];
'''PS:''' [[Pulmonary stenosis]];
'''AF:''' [[Atrial fibrillation]];
'''VF:''' [[Ventricular fibrillation]];
'''PR:''' [[Pulmonary regurgitation]];
'''RVOT:''' [[Right ventricular outflow tract]];
'''CMR:''' [[Cardiovascular magnetic resonance]];
'''SVC:''' [[Superior vena cava]];
'''IVC:''' [[Inferior vena cava]];
</span>
<br>


●Selection of surfactant preparation
{| style="cellpadding=0; cellspacing= 0; width: 600px;"
|-
| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF;" align=center |''' Recommendation for surgery in [[cyanotic congenital heart disease]]'''
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left | ''' Indications for repair of a scimitar vein in [[Anomalous pulmonary venous connection]] ([[TAPVC]])  ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]] ):'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Decreased functional capacity <br> 
❑ [[Right ventricle]] enlagment <br>
❑ Net left to right shunt or QP/QS > 1.5/1<br>
❑ [[Pulmonary artery ]]systolic pressure less than 50% systemic pressure<br>
❑ [[Pulmonary vascular resistance]] less than 1/3 of systemic resistance<br>
❑ Repair at the time of closure of a sinus venous defect or [[ASD]]<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red">Definition:</span>]] Abnormal connection between pulmonary veins and systemic veins leading to right heart volume overload such as ASD</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |''' Indications for [[surgery]] in  [[Anomalous Pulmonary Venous Connections]] ([[TAPVC]])'''([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence B]]) :'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic adults with [[right ventricle]] volume overload<br>
❑ Large left to right shunt( QP/QS > 1.5/1 <br>
❑ [[Pulmonary artery pressure]] <50% systemic pressure and [[pulmonary artery resistance]] <1/3 systemic resistance<br>
❑ Evidence of [[Right ventricle]] volume overload and QP/QS>1.5/1<br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |''' Indications for [[surgical]] repair or reoperation  in [[Ebstein anomaly]] : ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Significant [[tricuspid regurgitation]] in the presence of the following:<br>
❑ [[Heart failure ]] symptoms<br>
❑ Decreased [[functional capacity]]<br>
❑ Progressive [[right ventricular]] dysfunction by [[echocardiography]] or [[cardiac MRI]]<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red">Definition:</span>]] Malformation of tricuspid valve and right ventricle , atrialization of right ventricle, huge right atrium, accompanied by ASD, VSD, PS</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indications for [[surgical]] repair or reoperation in [[Ebstein anomaly]]:([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Significant [[tricuspid regurgitation ]] in the presence of the following:<br>
❑ Progressive [[right ventricle]] enlargement<br>
❑ Systemic desaturation due to [[right to left shunt]] via [[ASD]], [[VSD]]<br>
❑ [[Paradoxical emboli]] through [[ASD]], [[VSD]]<br>
❑ [[Atrial tachycardia]]<br>
<span style="font-size:85%;color:red"> [[Other surgery procedures|<span style="color:red"> Other surgery procedures:</span>]] Closure ASD, ablation of multiple accessory pathway for prevention of VF, left atrium COX-MAZ 3  in the presence of AF</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for Glenn anastomousis at the time of repair in [[Ebstein anomaly]] : ([[ACC AHA guidelines classification scheme|Class 2b, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Severe [[right ventricle]] dilation <br>
❑ Severe [[right ventricular systolic dysfunction]]<br>
❑ Normal [[left ventricle]] function<br>
❑ Normal [[left atrium]] or [[left ventricle end diastolic pressure]]<br>
<span style="font-size:85%;color:red"> [[Glenn shunt|<span style="color:red"> Glenn anastomosis:</span>]] Bidirectional superior cavopulmonary anastomosis which is the connection between superior vena cava and pulmonary artery with bypassing right artium and right ventricle, cardiac catheterization should be done before glenn anastomosis especially in adult with hypertension  for evaluation of left ventricle diastolic pressure. </span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left | ''' Indication for [[pulmonary valve replacement ]] ( surgical or percutaneous in [[Tetralogy of fallot]] ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]]):'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑  Symptomatic Moderate to severe [[pulmonary regurgitation]] after repaired [[TOF]] in which symptoms can not be explained otherwise.<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red">Definition:</span>]] The combination of Right ventricle hypertrophy, VSD, PS, Overridding aorta</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Pulmonary valve replacement ]] ( surgical or percutaneous in [[Tetralogy of fallot]] : ([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic moderate to severe [[ pulmonary regurgitation]] after repaired [[TOF]]<br>
<span style="font-size:85%;color:red"> Pulmonary stenosis valvotomy:<span style="color:red"> Surgical or balloon valvotomy in case of severe PS during infancy or childhood is recommended. Both of interventions can result pulmonary regurgitation and right ventricle dilation  in the future and the need for pulmonary valve replacement.</span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Pulmonary valve replacement ]] (surgical) in [[Tetralogy of fallot]] : ([[ACC AHA guidelines classification scheme|Class 2b, Level of Evidence C]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Moderate to severe [[PR]] with other lesions requiring surgery in repaired [[TOF]]<br>
❑ Moderate to severe [[PR]] with ventricular tachycardia requiring arrhythmia management in repaired [[TOF]]<br>
<span style="font-size:85%;color:red"> [[Other lesions requiring surgery|<span style="color:red"> Other lesions requiring surgery:</span>]] RVOT aneurysm, TR, branch PA stenosis, residual VSD, arrhythmia ablation, coronary artery revascularization, aortic root replacement </span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Tricuspid valve replacement ]] in  [[CCTGA]] : ([[ACC AHA guidelines classification scheme|Class I, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Symptomatic severe [[tricuspid regurgitaion]] accompanied by preserved or mildly systolic dysfunction of systemic ventricle<br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Tricuspid valve replacement ]] in  [[CCTGA]] : ([[ACC AHA guidelines classification scheme|Class 2a, Level of Evidence C]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic  severe [[tricuspid regurgitation]] accompanied by mildly dilated systemic [[ventricle]]<br>
<span style="font-size:85%;color:red"> [[Definition|<span style="color:red"> Definition:</span>]] Atrioventricular discordance and ventriculoarterial discordance leading to physiologic corrected circulation accompanied by VSD(75%), pulmonary or subpulmonary stenosis(75%),left sided tricuspid and Ebstein like valve anomalies(75%) </span><br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for conduit intervention/replacement in  [[CCTGA]] : ([[ACC AHA guidelines classification scheme|Class 2b, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Symptomatic subpulmonary [[left ventricle]] to [[pulmonary artery]] conduit dysfunction <br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Balloon valvoplasty ]] in  [[Pulmonary stenosis]] : ([[ACC AHA guidelines classification scheme|Class I , Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ In adult with moderate to severe  [[valvular PS]] with symptoms of [[heart failure]], [[cyanosis]] from intracardiac  right to left [[shunt]], [[exercise intolerance]]<br>
❑ Surgical repair is recommended if balloon valvoplasty  in indicated patients failed <br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Balloon valvoplasty]] in  [[Pulmonary Stenosis]] : ([[ACC AHA guidelines classification scheme|Class 2a , Level of Evidence C]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ Asymptomatic severe [[valvular PS]]<br>
|-
|style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Indication for [[Dilation and Stenting]] in [[Peripheral Pulmonary Stenosis]] : ([[ACC AHA guidelines classification scheme|Class  2a, Level of Evidence B]])'''
|-
|style="padding: 0 5px; font-size: 100%; background: #F5F5F5; width: 70%" align=left|
❑ In an adult with branch and peripheral [[PS]], [[pulmonary artery]] dilation  and stenting is recommended
|}


●Indications for surfactant therapy
== Surgical procedures in [[d-TGA]] and [[Tricuspid Atresia]] ==


●Timing of administration


●Technical aspects of administration
{| class="wikitable"
|-
! Surgical management in [[d-TGA]] || Surgical management in [[Tricuspid Atresia]]
|-
! Life-saving balloon atrial septostomy in neonatal period||Blalock Taussig shunt
|-
|Connection  between right and the left system is necessary for the life
||                                                   
*In the first 8 weeks of birth  for transferring the systemic  blood to the pulmonary circulation in the neonate with cyanosia and pulmonary obstruction and normal positioning [[aorta]] and [[pulmonary artery]]<ref name="pmid26260095">{{cite journal |vauthors=Aykanat A, Yavuz T, Özalkaya E, Topçuoğlu S, Ovalı F, Karatekin G |title=Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease |journal=Pediatr Cardiol |volume=37 |issue=1 |pages=131–4 |date=January 2016 |pmid=26260095 |doi=10.1007/s00246-015-1251-0 |url=}}</ref><span style="font-size:85%;color:red"> [[Definition|<span style="color:red"> Definition:</span>]] Absence of trisuspid valve and right ventricle hypoplasia,connection via ASD is necessary </span><br>
|-
! Atrial switch procedure|| [[Pulmonary artery]] banding.<ref name="pmid30811802">{{cite journal |vauthors=Boucek DM, Qureshi AM, Goldstein BH, Petit CJ, Glatz AC |title=Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature |journal=Congenit Heart Dis |volume=14 |issue=1 |pages=105–109 |date=January 2019 |pmid=30811802 |doi=10.1111/chd.12707 |url=}}</ref>
|-
| 
* Mustard  procedure (baffle made of Dacron or [[pericardium]]) or [[Senning]] procedure(atrial flap)<ref name="VejlstrupSørensen2015">{{cite journal|last1=Vejlstrup|first1=Niels|last2=Sørensen|first2=Keld|last3=Mattsson|first3=Eva|last4=Thilén|first4=Ulf|last5=Kvidal|first5=Per|last6=Johansson|first6=Bengt|last7=Iversen|first7=Kasper|last8=Søndergaard|first8=Lars|last9=Dellborg|first9=Mikael|last10=Eriksson|first10=Peter|title=Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark|journal=Circulation|volume=132|issue=8|year=2015|pages=633–638|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.114.010770}}</ref>


Types of surfactant
* The most common procedure in older patients
* Blood is diverted at the atrial level into the physiologic ventricle
* Systemic venous return is diverted into [[left ventricle]] through [[mitral valve]]
* Pulmonary venous return is directed into [[right ventricle]] through [[tricuspid valve]]
<span style="font-size:85%;color:red"> [[d-TGA definition|<span style="color:red"> d-TGA definition:</span>]] Aorta arises  from right ventricle and pulmonary artery arises from left ventricle </span><br>
||
* Useful in  overflow [[pulmonary artery]] coming from [[left ventricle]] for lowering the [[pulmonary blood flow]]
|-
! Arterial switch procedure || Bidirectional Glenn shunt
|-
|                           
*Transection and anastomoses of [[aorta]] and [[pulmonary artery ]] to the contralateral ventricle
* Closure of the [[VSD]]
* Transposioning of the [[coronary artrie]]
* Benefit is the restoration of [[left ventricular]] systolic function
||
*Useful in older children for blood transferring from [[SVC]] to [[pulmonary artery]]
|-
! Rastelli procedure|| Fontan procedure<ref name="pmid8238751">{{cite journal |vauthors=Norwood WI, Jacobs ML |title=Fontan's procedure in two stages |journal=Am. J. Surg. |volume=166 |issue=5 |pages=548–51 |date=November 1993 |pmid=8238751 |doi=10.1016/s0002-9610(05)81151-1 |url=}}</ref><ref name="d'UdekemIyengar2007">{{cite journal|last1=d'Udekem|first1=Y.|last2=Iyengar|first2=A. J.|last3=Cochrane|first3=A. D.|last4=Grigg|first4=L. E.|last5=Ramsay|first5=J. M.|last6=Wheaton|first6=G. R.|last7=Penny|first7=D. J.|last8=Brizard|first8=C. P.|title=The Fontan Procedure: Contemporary Techniques Have Improved Long-Term Outcomes|journal=Circulation|volume=116|issue=11_suppl|year=2007|pages=I-157–I-164|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.106.676445}}</ref>


Surfactant preparations include natural and synthetic surfactants. Although both types of surfactant preparations are effective, natural surfactants have been shown to be superior in clinical trials to synthetic preparations that did not contain protein B and C analogues [3,33,34]. In particular, the use of natural preparations was associated with lower inspired oxygen concentration and ventilator pressures, decreased mortality, and lower rate of RDS complications in preterm infants.


Three natural surfactants derived from either bovine or porcine lungs are commercially available in the United States (table 1). It appears that there are no clinically significant differences amongst the three preparations [35,36]:
|-
|
* Systemic to pulmonary artery shunt when [[pulmonary blood flow]] decreases<ref>{{cite journal|doi=10.1177/2F2150135118817765}}</ref>


●Poractant alfa – Porcine lung minced extract
* Useful in infants with [[TGA]] and [[VSD]] and [[PS]]
*Function: Re-direction of ventricular outflow
*Placement a conduit between [[right ventricle]] and [[pulmonary artery]] and also a baffle between [[left ventricle]] and [[aorta]]
* Survival rate is poor compared with mustard and arterial switch
||
*Useful in older children aged 2-3
* A conduit for transferring  blood from [[IVC]] to [[pulmonary artery]]
|-
|}


●Calfactant – Bovine lung lavage extract
== Palliative Systemic-to-Pulmonary shunts ==
{| class="wikitable"
|-
! Arterial
|-
| [[Blalock-taussing-Thomas shunt]] ([[subclavian artery]] to [[pulmonary artery]])
|-
| [[Central shunt]] ([[aorta]] to [[pulmonary artery]])
|-
| [[Potts shunt]] (descending aorta to [[left pulmonary artery]]
|-
| [[Waterston shunt]] ([[Ascending aorta]] to [[right pulmonary artery]])
|-
! Venous
|-
| Glenn shunt ([[SVC]] to the ipsilateral [[pulmonary artery]])
|-
| Bidirectional cavopulmonary (Glenn) shunt( end to side [[SVC]]  to [[left pulmonary artery]]  and [[right pulmonary artery]] shunt)
|}


●Beractant – Bovine lung minced extract
* [[Right ventricle]]–to-[[pulmonary artery]] conduits is recommended in severe [[RVOT]] obstruction such as [[pulmonary atresia]].
* theses conduits may be homografts or prosthetic conduits with bioprosthetic valves using within the conduit.
*Common complications of the conduits may include the following:
:*Kinking
:*[[Aneurysmal dilation]]
:*Conduit dysfunction over time
:*Progressive stenosis within the [[conduit]] or at the [[valve]]
:*Valvular [[regurgitation]]


Additional doses of surfactant therapy are administered if the patient has a persistent requirement of an FiO2 >0.30. Subsequent surfactant administration may decrease mortality and morbidity in infants less than 30 weeks gestation with RDS [27,39].
If the infant maintains adequate respiratory efforts and has an FiO2 requirement less than 0.30, no additional doses of surfactant are needed and the patient can be extubated to nCPAP [27,39].
Timing — If surfactant therapy is used, it is most effective when given within the first 30 to 60 minutes of life following placement of a pulse oximeter and clinical confirmation of correct endotracheal tube placement. However, the potential benefits of timely administration of surfactant must be balanced with adequate time for an initial trial of nCPAP [27,40,41].
Surfactant administration technique
Endotracheal administration — Endotracheal intubation has been the standard technique of surfactant administration. However, surfactant administration may be complicated by transient airway obstruction [3,42] or inadvertent instillation into only the right main stem bronchus if the endotracheal tube is advanced too far in the airway. During administration, oxygen saturation needs to be monitored, as oxygen desaturation may occur. Other complications associated with intubation and mechanical ventilation include pulmonary injury due to volutrauma and barotrauma associated with intermittent positive pressure ventilation, pulmonary air leak, and airway injury due to intubation. (See 'Endotracheal tube complications' below.)
Less invasive measures — Due to the complications from the delivery of surfactant by intubation, minimal or less invasive administrative techniques have been developed and appear promising. These interventions include aerosolized surfactant preparations, laryngeal mask airway-aided delivery of surfactant, pharyngeal instillation, and the use of thin intratracheal catheters [43-49].
However, evidence is of moderate quality that support their use over the traditional endotracheal administration. As a result, we continue to administer surfactant through the endotracheal route until there are conclusive data of the effectiveness, safety, and generalizability of these new noninvasive techniques. The use of less invasive measures to administer surfactant has expanded, especially in European centers [50]. However, there is wide variation in the administration and techniques used and in patient selection.
Surfactant in combination with budesonide
Limited data in preterm infants with severe RDS requiring mechanical ventilation suggest that the combination of surfactant and budesonide(corticosteroid) reduced the incidence of BPD and the composite outcome of death and BPD [55]. There was no difference in mortality. However, there were several limitations raising concern of bias, including small number of patients, studies performed by the same group, incomplete blindness in the study design, and follow-up of the entire cohort at two to three years of age. As a result, the combination of surfactant and budesonide cannot be recommended until there are larger studies that show definite benefit that outweighs any adverse effect of the intervention.
Inhaled nitric oxide
Data from clinical trials show that the use of inhaled nitric oxide (iNO) either as rescue or routine therapy is not beneficial in preterm infants with RDS in reducing mortality or the risk of BPD. As a result, we concur with the 2014 AAP clinical report and the ECG guidelines that iNO should not be used to treat preterm infants with RDS except in rare cases of pulmonary hypertension or hypoplasia [2,56]. The evidence is discussed in greater detail separately.
Thermoregulation
Infants should be maintained in a thermal neutral environment to minimize heat loss and maintain the core body temperature in a normal range, thereby reducing oxygen consumption and caloric needs. The ambient temperature should be selected to maintain an anterior abdominal skin temperature in the 36.5 to 37ºC range. Rectal temperatures should be avoided in infants with RDS because of the greater risk of trauma or perforation associated with their use. As a result, abdominal temperatures are used to set the servo-controlling temperatures in incubators and in radiant warmers. (See "Short-term complications of the preterm infant", section on 'Hypothermia'.)
Fluid management
Fluids should be adjusted to maintain a slightly negative water balance, as infants are born in a positive fluid state. Excessive fluid intake may increase the risk of patent ductus arteriosus (PDA), necrotizing enterocolitis (NEC), and bronchopulmonary dysplasia (BPD) [57] and should be avoided.
There is no evidence to support the routine use of diuretics (particularly furosemide) in preterm infants with RDS [58]. Diuretic use should be avoided because it often results in serum electrolyte abnormalities, and in the case of loop diuretics, nephrocalcinosis, especially hyponatremia and hypokalemia, due to urinary loss of sodium and potassium. (See "Fluid and electrolyte therapy in newborns", section on 'Disorders of sodium balance'.)
Cardiovascular management
Cardiovascular management is focused on ensuring adequate perfusion for all patients. Systemic hypotension occurs commonly in the early stages of RDS. As a result, blood pressure should be frequently monitored noninvasively or continuously via intravascular catheter. However, intervention is not usually required for extremely low birth weight (ELBW) infants (BW <1000 g) with adequate perfusion. In contrast, infants with poor perfusion are in shock and require resuscitation to stabilize their hemodynamic state. (See "Etiology, clinical manifestations, evaluation, and management of low blood pressure in extremely preterm infants", section on 'Management approach' and "Etiology, clinical manifestations, evaluation, and management of neonatal shock".)
PDA is common in preterm infants with RDS. It may manifest as hypercapnia and contribute to difficulties in weaning from mechanical ventilation, which may predispose the patient to BPD. The clinical features, diagnosis, and management of PDA in preterm infants are discussed separately. (See "Pathophysiology, clinical manifestations, and diagnosis of patent ductus arteriosus in premature infants" and "Management of patent ductus arteriosus in preterm infants".)
== Pulmonary atresia ==
* Cardiac catheterization is done to evaluate the defect or defects of the heart; this procedure is much more invasive.
* The patient will need to have a series of surgeries to improve the blood flow permanently.
* The type of surgery recommended depends on the size of the [[right ventricle]] and the [[pulmonary artery]]. If they are normal in size and the right ventricle is able to pump blood, [[open heart surgery]] can be performed to make blood flow through the heart in a normal pattern. If the right ventricle is small and unable to act as a pump, doctors may perform another type of operation called the [[Fontan procedure]]. In this two-stage procedure, the [[right atrium]] is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, by-passing the heart.  The first surgery will likely be performed shortly after birth. A [[shunt]] can be created between the aorta and the pulmonary artery to help increase blood flow to the lungs. As the child grows, so does the heart and the shunt may need revised in order to meet the body's requirements.
* A cardiac catheterization procedure can be used as a diagnostic procedure, as well as initial treatment procedure as balloon atrial septostomy to improve mixing oxygenated blood and unoxygenated blood between the right and left atria.
* '''Atrial Septostomy''': a special catheter with a balloon in the tip is used to create an opening in the atrial septum. The catheter is guided through the foramen ovale to the left atrium (LA). Once the ballon is in the LA is inflated and then pulled back opening a bigger hole between the right atrium and the LA to mix blood.
* If the hospital does not have a catheterization lab with skill physician to perform the ballon atrial septostomy, an intravenous medication called prostaglandin is administered to keep the ductus arteriousus from closing.
== Transposition of great arteries ==
'''Palliative interventions'''
*Cardiac catheterization
**Rashkind balloon atrial septostomy
**Balloon angioplasty
**Endovascular stenting
**Angiography
'''Cardiac catheterization''' is a [[minimally invasive procedure]] which provides a means of performing a number of other procedures.
*A '''balloon atrial septostomy''' is performed with a balloon catheter, which is inserted into a foramen ovale, PFO, or ASD and inflated to enlarge the opening in the atrial septum; this creates a shunt which allows a larger amount of red blood to enter the systemic circulation.
*Angioplasty also requires a balloon catheter, which is used to stretch open a stenotic vessel; this relieves restricted blood flow, which could otherwise lead to CHF.
*An '''endovascular stent''' is sometimes placed in a stenotic vessel immediately following a balloon angioplasty to maintain the widened passage.
*Angiography involves using the catheter to release a contrast medium into the chambers and/or vessels of the heart; this process facilitates examining the flow of blood through the chambers during an echocardiogram, or shows the vessels clearly on a chest x-ray, MRI, or CT scan - this is of particular importance, as the coronary arteries must be carefully examined and "mapped out" prior to the corrective surgery.
It is commonplace for any of these palliations to be performed on a TGA patient.
'''Moderate'''
*Left anterior thoracotomy
**Isolated pulmonary artery banding (PAB)
*Left lateral thoracotomy
**PAB (when coarctation or aortic arch repair also required)
*Right lateral thoracotomy
**Blalock-Hanlon atrial septectomy
Each of these procedures are performed through an incision between the ribs and visualized by echocardiogram; these are far less common than heart cath procedures.
Pulmonary artery banding is used in a small number of cases of d-TGA, usually when the corrective surgery needs to be delayed, to create an artificial stenosis in order to control pulmonary blood pressure; PAB involves placing a band around the pulmonary trunk, this band can then be quickly and easily adjusted when necessary.
An atrial septectomy is the surgical removal of the atrial septum; this is performed when a foramen ovale, PFO, or ASD are not present and additional shunting is required to raise the oxygen saturation of the blood.
'''Major'''
*Median sternotomy
**PAB (when intracardiac procedures also required)
**Concomitant atrial septectomy
In recent years, it is quite rare for palliative procedures to be done via median sternotomy. However, if a sternotomy is required for a different procedure, in most cases all procedures that are immediately required will be performed at the same time.
== Total anomalus pulmonary venous return ==
Surgery should be performed as soon as possible in the patients of total anomalous pulmonary venous connection. The surgical procedure varies depending upon the anatomy of the TAPVC lesion.
*Supracardiac and infracardiac TAPVC: Connection is created between the [[Pulmonary vein|pulmonary veins]] and the [[left atrium]]. The vertical vein is tied up so that the abnormal blood flow could be prevented.
*Pulmonary veins directly connected to the superiorvenacava: An intracardiac baffle is created that helps in transfer of blood from the [[right atrium]], through atrial septum into the left atrium.
*Intracardiac (pulmonary vein connected to the coronary sinus): Coronary sinus is incised and connected to left atrium.
*Intracardiac (pulmonary vein opening directly into the right atrium): A interatrial connection is made and the blood is redirected from right atrium to left atrium.
== Tricuspid atresia ==
First stage
First stage of surgery is performed in the neonatal period. The goals of the initial palliation are to ensure that blood exiting the right atrium (RA) is unimpeded, provides adequate pulmonary blood flow, protects the pulmonary artery bed from high pressures that could result in higher risk for subsequent operations, and ensures unobstructed flow from the left ventricle (LV) to the aorta. Choices for intervention are dependent on the anatomic variants as follows (table 1):
●TV atresia with normally related great arteries (type I):
•With diminished pulmonary blood flow, the initial surgery soon after birth aims to restore a reliable source of pulmonary blood flow with a modified Blalock-Taussig shunt (shunt from the innominate artery into the central pulmonary artery) (figure 4).
•In the presence of unobstructed pulmonary blood flow (type Ic), a pulmonary artery band may be placed in early infancy to restrict the amount of pulmonary blood flow and protect the pulmonary bed from high systemic pressures. As mentioned above, the vast majority of ventricular septal defects (VSDs) decrease in size over time and restrict pulmonary blood flow. A pulmonary artery band, therefore, may not be required in all patients who have unobstructed pulmonary flow at birth through a VSD, and could be restricted to patients who are symptomatic despite maximal medical therapy for pulmonary congestion/heart failure. Furthermore, a pulmonary artery band may stimulate myocardial hypertrophy that may more rapidly reduce the size of the VSD.
•Rarely, the degree of restriction to pulmonary blood flow is enough to maintain adequate oxygenation without pulmonary overcirculation and heart failure. These patients may not require surgery in the neonatal period and can be taken directly to the second stage of palliation.
●TV atresia with transposition of great arteries (type II):
•In the presence of significant subaortic obstruction with a restrictive VSD, enlargement of the VSD or a Damus-Kaye-Stansel anastomosis (anastomosis between the main pulmonary artery and ascending aorta) with a modified Blalock-Taussig shunt is the initial surgery of choice.
•If an isolated coarctation is present, it should be relieved and a pulmonary artery band may be considered to restrict pulmonary blood flow.
•Similar to type I lesions, in a few cases, the size of the VSD is large enough to maintain systemic output, in which case a pulmonary artery band alone may be adequate.
Second stage — The second palliative procedure for both type I and type II lesions is a cavopulmonary anastomosis (Glenn procedure). This stage of palliation is typically performed at three to six months of life when infants experience progressive cyanosis as they begin to outgrow their neonatal shunt. This surgery involves removal of the original shunt, and direct anastomosis of the superior vena cava to the right pulmonary artery. The Glenn procedure relies on passive venous drainage from the superior vena cava directly into the pulmonary artery. However, there is persistent systemic desaturation due to continued inferior vena cava flow into the RA. In a case series of 557 patients who underwent second stage palliation for single ventricle anatomy from 1998 to 2010, the overall mortality rate was low (4.7 percent) and there were no deaths in the 12 percent of patients who had TV atresia [25]
== Tetralogy of fallot ==
=== Palliative surgery ===
*The surgery involved forming an [[anastomosis]] between the [[subclavian artery]] and the [[pulmonary artery]].
*The Pott shunt and the Waterson procedure are other shunt procedures which were developed for the same purpose.
*Potts shunt: Shunt between [[left pulmonary artery]] and [[descending aorta]].
*The technique has been modified and is usually performed using a Gortex tube to create the connection.
*Waterson shunt: Shunt between [[right pulmonary artery]] and [[ascending aorta]].
===Total Surgical Repair===
*The surgery generally involves:
**Making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle
**Repairing the VSD using a Gore-Tex or Dacron patch or a homograft.
**Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.
**The repair could be done by either of the approaches i.e.transatrial or transpulmonary
== Coarcitation of aorta ==
===Indications for Surgery===
*There is a lack of consensus for the indications and the time for surgery. Some groups of surgeon consider [[balloon angioplasty]] and stenting as the initial approach and reserving surgery for more complicated conditions or treatment failures. Whereas, others consider surgical repair as the first line of therapy.
*Surgery is the first choice in aortic coarctations involving:
**Long length of aorta
**Severe coarctations causing almost complete or complete obliteration of the aortic lumen.
**Associated with other cardiac defects for e.g. large [[patent ductus arteriosus]], [[ventricular septal defect]].
=== Surgical Techniques===
*The choice of technique depends on the patient's age at presentation, size, associated abnormalities, and anatomy of the coarctation.
*Surgical approach - [[median sternotomy]] is preferred over left lateral thoracotomy, in complex arch repairs.
*Subclavian flap aortoplasty is found to be the most commonly performed followed by resection in end-to-end anastomosis, patch aortoplasty, and bypass graft when the surgery is done during infancy. Whereas, in children and adolescent undergoing coarctation repair end-to-end anastomosis is commonest followed patch aortoplasty and subclavian flap aortoplasty.
*Patch aortoplasty are less frequently used these days because of concerns regarding the development of aortic aneurysm at the site of surgery. 
*Available Techniques:
**Resection and end-to-end anastomosis
**Patch aortoplasty
**Left subclavian flap aortoplasty
**Tubular bypass grafts
**Combination techniques 
== Atrial septal defect ==
*Surgical closure is the most common method of treatment method for [[atrial septal defect]] and has been the gold standard for many years. Many surgeons prefer more [[Minimally invasive surgery|minimally invasive techniques]] over the conventional [[sternotomy]] to avoid potentials for additional complications. Special consideration must be taken into account for the age of the patient and the size of the defect involved. Surgical closure is indicated for patients with [[Atrial septal defect ostium primum|primum]], [[Atrial septal defect sinus venosus|sinus venosus]] and [[Atrial septal defect coronary sinus|coronary sinus]] type of [[Atrial septal defect|atrial septal defects]]. However, ostium secundum atrial septal defects are commonly treated by percutaneous closure. With uncomplicated [[atrial septal defect]], (without [[pulmonary hypertension]] and other comorbidities) the post-surgical mortality is as low as 1%.  Minimally invasive repair of [[atrial septal defect]] has been shown to be as successful as the conventional [[sternotomy]]. Although they have not been associated with reduced morbidity and mortality rates, they have been proven to have the advantage of being less invasive, less post-surgical complications, decreased hospital stay, and more cosmetic benefits.
== Pulmonary hypertension ==
*The choice of treatment for pulmonary hypertension requires the assessment of the clinical severity of the disease and the identification of any underlying cause.
*Patients who have PH secondary to a medical condition such as [[left heart failure]], [[lung]] diseases, or [[Thromboembolism|thromboembolic disease]] should receive treatment for the underlying cause.
*Patients who have pulmonary arterial hypertension (PAH) must undergo vasoreactivity testing in order to assist in the selection of the optimal therapy which includes [[Calcium channel blocker|calcium channel blockers]], [[endothelin receptor antagonist]], [[Phosphodiesterase inhibitor|phosphodiesterase inhibitors]], or [[Prostanoid|prostanoids]].
*Surgical intervention such as [[atrial septostomy]] or [[lung transplantation]] should be considered among patients with pulmonary arterial hypertension who fail to improve on optimal therapy or when medical therapy is unavailable.
*Failure of clinical improvement among PAH patients with [[Pulmonary hypertension classification#WHO Functional Classification|WHO functional class II or III]] is defined as either:
**A stable and unsatisfactory clinical status, or
**An unstable and deteriorating Failure of clinical improvement among PAH patients with [[Pulmonary hypertension classification#WHO Functional Classification|WHO functional class IV]] is defined as either:
**Absence of quick improvement to a WHO functional class III or less, or
**A stable and unsatisfactory clinical status
===Atrial Septostomy===
*[[Atrial septostomy]] is a surgical procedure that creates a shunt between the right and left [[atria]].
*It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood ([[hypoxia]]). It is best performed in experienced centers.
*Graded balloon dilatation of atrial septostomy is the recommended type of [[atrial septostomy]].
*Atrial septostomy might be considered among PH patients with [[Pulmonary hypertension classification#WHO Functional Classification|WHO functional class IV]] and [[right heart failure]] refractory to therapy.
*[[Atrial septostomy]] should also be considered among PH patients who have [[Eisenmenger's syndrome]], idiopathic PAH, and those awaiting [[lung transplantation]].
===Lung Transplantation===
*[[Lung transplantation]] is considered in the treatment of patients with idiopathic PH, PH associated with [[congenital heart disease]], or pulmonary veno-occlusive disease (PVOD) who fail to improve on optimal medical therapy.
*Combined lung and heart transplantation might be considered in selected patients.
*According to the Registry of the International Society for Heart and Lung Transplantation, the survival rates following [[lung transplantation]] are 61%, 49%, and 25 % at 3, 5, and 10 years respectively.
===Pulmonary Thromboendarterectomy===
*[[Pulmonary thromboendarterectomy]] (PTE) is a surgical procedure that is used for the treatment of chronic thromboembolic pulmonary hypertension.
*It is the surgical removal of an organized [[thrombus]] along with the lining of the [[pulmonary artery]].
*PTE is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
*Treatment for hypoxic and miscellaneous varieties of PH have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
 
[[Category:Up To Date]]
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{{WS}}
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Zand, M.D.[2]

Overview

Cardiac defects causing central cyanosis include: Transposition of the great arteries, Tetralogy of fallot, Tricuspid atresia, Truncus arteriosus,Total anomalous pulmonary venous connection, Ebstein anomaly, critical Pulmonary stenosis or atresia, functional single ventricle. The palliative surgical shunt maybe done in such lesions to increase pulmonary blood flow even in the presence of cyanosis. Complete repair procedure leads to relief of cyanosis and shunt and also has long term complications.

Recommendation for surgery in cyanotic heart disease

The table shows indications for surgery in cyanotic congenital heart disease according to 2018 AHA/ACC Guideline:[1] [2][3][4][5]


Abbreviations: d-TGA: dextro-Transposition of great arteries; PDA: Patent ductus arteriosus  ; ASD: Atrial septal defect; VSD: Ventricular septal defect; TAPVC: Total anomalous pulmonary venous connection; TOF: Tetralogy of fallot; CCTGA: Congenitally corrected transposition of the great arteries; PS: Pulmonary stenosis; AF: Atrial fibrillation; VF: Ventricular fibrillation; PR: Pulmonary regurgitation; RVOT: Right ventricular outflow tract; CMR: Cardiovascular magnetic resonance; SVC: Superior vena cava; IVC: Inferior vena cava;

Recommendation for surgery in cyanotic congenital heart disease
Indications for repair of a scimitar vein in Anomalous pulmonary venous connection (TAPVC) (Class I, Level of Evidence B ):

❑ Decreased functional capacity
❑ Right ventricle enlagment
❑ Net left to right shunt or QP/QS > 1.5/1
❑ Pulmonary artery systolic pressure less than 50% systemic pressure
❑ Pulmonary vascular resistance less than 1/3 of systemic resistance
❑ Repair at the time of closure of a sinus venous defect or ASD
Definition: Abnormal connection between pulmonary veins and systemic veins leading to right heart volume overload such as ASD

Indications for surgery in Anomalous Pulmonary Venous Connections (TAPVC)(Class 2a, Level of Evidence B) :

❑ Asymptomatic adults with right ventricle volume overload
❑ Large left to right shunt( QP/QS > 1.5/1
❑ Pulmonary artery pressure <50% systemic pressure and pulmonary artery resistance <1/3 systemic resistance
❑ Evidence of Right ventricle volume overload and QP/QS>1.5/1

Indications for surgical repair or reoperation in Ebstein anomaly : (Class I, Level of Evidence B)

❑ Significant tricuspid regurgitation in the presence of the following:
❑ Heart failure symptoms
❑ Decreased functional capacity
❑ Progressive right ventricular dysfunction by echocardiography or cardiac MRI
Definition: Malformation of tricuspid valve and right ventricle , atrialization of right ventricle, huge right atrium, accompanied by ASD, VSD, PS

Indications for surgical repair or reoperation in Ebstein anomaly:(Class 2a, Level of Evidence B)

❑ Significant tricuspid regurgitation in the presence of the following:
❑ Progressive right ventricle enlargement
❑ Systemic desaturation due to right to left shunt via ASD, VSD
❑ Paradoxical emboli through ASD, VSD
❑ Atrial tachycardia
Other surgery procedures: Closure ASD, ablation of multiple accessory pathway for prevention of VF, left atrium COX-MAZ 3 in the presence of AF

Indication for Glenn anastomousis at the time of repair in Ebstein anomaly : (Class 2b, Level of Evidence B)

❑ Severe right ventricle dilation
❑ Severe right ventricular systolic dysfunction
❑ Normal left ventricle function
❑ Normal left atrium or left ventricle end diastolic pressure
Glenn anastomosis: Bidirectional superior cavopulmonary anastomosis which is the connection between superior vena cava and pulmonary artery with bypassing right artium and right ventricle, cardiac catheterization should be done before glenn anastomosis especially in adult with hypertension for evaluation of left ventricle diastolic pressure.

Indication for pulmonary valve replacement ( surgical or percutaneous in Tetralogy of fallot (Class I, Level of Evidence B):

❑ Symptomatic Moderate to severe pulmonary regurgitation after repaired TOF in which symptoms can not be explained otherwise.
Definition: The combination of Right ventricle hypertrophy, VSD, PS, Overridding aorta

Indication for Pulmonary valve replacement ( surgical or percutaneous in Tetralogy of fallot : (Class 2a, Level of Evidence B)

❑ Asymptomatic moderate to severe pulmonary regurgitation after repaired TOF
Pulmonary stenosis valvotomy: Surgical or balloon valvotomy in case of severe PS during infancy or childhood is recommended. Both of interventions can result pulmonary regurgitation and right ventricle dilation in the future and the need for pulmonary valve replacement.

Indication for Pulmonary valve replacement (surgical) in Tetralogy of fallot : (Class 2b, Level of Evidence C)

❑ Moderate to severe PR with other lesions requiring surgery in repaired TOF
❑ Moderate to severe PR with ventricular tachycardia requiring arrhythmia management in repaired TOF
Other lesions requiring surgery: RVOT aneurysm, TR, branch PA stenosis, residual VSD, arrhythmia ablation, coronary artery revascularization, aortic root replacement

Indication for Tricuspid valve replacement in CCTGA : (Class I, Level of Evidence B)

❑ Symptomatic severe tricuspid regurgitaion accompanied by preserved or mildly systolic dysfunction of systemic ventricle

Indication for Tricuspid valve replacement in CCTGA : (Class 2a, Level of Evidence C)

❑ Asymptomatic severe tricuspid regurgitation accompanied by mildly dilated systemic ventricle
Definition: Atrioventricular discordance and ventriculoarterial discordance leading to physiologic corrected circulation accompanied by VSD(75%), pulmonary or subpulmonary stenosis(75%),left sided tricuspid and Ebstein like valve anomalies(75%)

Indication for conduit intervention/replacement in CCTGA : (Class 2b, Level of Evidence B)

❑ Symptomatic subpulmonary left ventricle to pulmonary artery conduit dysfunction

Indication for Balloon valvoplasty in Pulmonary stenosis : (Class I , Level of Evidence B)

❑ In adult with moderate to severe valvular PS with symptoms of heart failure, cyanosis from intracardiac right to left shunt, exercise intolerance
❑ Surgical repair is recommended if balloon valvoplasty in indicated patients failed

Indication for Balloon valvoplasty in Pulmonary Stenosis : (Class 2a , Level of Evidence C)

❑ Asymptomatic severe valvular PS

Indication for Dilation and Stenting in Peripheral Pulmonary Stenosis : (Class 2a, Level of Evidence B)

❑ In an adult with branch and peripheral PS, pulmonary artery dilation and stenting is recommended

Surgical procedures in d-TGA and Tricuspid Atresia

Surgical management in d-TGA Surgical management in Tricuspid Atresia
Life-saving balloon atrial septostomy in neonatal period Blalock Taussig shunt
Connection between right and the left system is necessary for the life
  • In the first 8 weeks of birth for transferring the systemic blood to the pulmonary circulation in the neonate with cyanosia and pulmonary obstruction and normal positioning aorta and pulmonary artery[6] Definition: Absence of trisuspid valve and right ventricle hypoplasia,connection via ASD is necessary
Atrial switch procedure Pulmonary artery banding.[7]

d-TGA definition: Aorta arises from right ventricle and pulmonary artery arises from left ventricle

Arterial switch procedure Bidirectional Glenn shunt
Rastelli procedure Fontan procedure[9][10]


  • Useful in older children aged 2-3
  • A conduit for transferring blood from IVC to pulmonary artery

Palliative Systemic-to-Pulmonary shunts

Arterial
Blalock-taussing-Thomas shunt (subclavian artery to pulmonary artery)
Central shunt (aorta to pulmonary artery)
Potts shunt (descending aorta to left pulmonary artery
Waterston shunt (Ascending aorta to right pulmonary artery)
Venous
Glenn shunt (SVC to the ipsilateral pulmonary artery)
Bidirectional cavopulmonary (Glenn) shunt( end to side SVC to left pulmonary artery and right pulmonary artery shunt)
  • Right ventricle–to-pulmonary artery conduits is recommended in severe RVOT obstruction such as pulmonary atresia.
  • theses conduits may be homografts or prosthetic conduits with bioprosthetic valves using within the conduit.
  • Common complications of the conduits may include the following:

References

  1. ↑ Stout, Karen K.; Daniels, Curt J.; Aboulhosn, Jamil A.; Bozkurt, Biykem; Broberg, Craig S.; Colman, Jack M.; Crumb, Stephen R.; Dearani, Joseph A.; Fuller, Stephanie; Gurvitz, Michelle; Khairy, Paul; Landzberg, Michael J.; Saidi, Arwa; Valente, Anne Marie; Van Hare, George F. (2019). "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines". Circulation. 139 (14). doi:10.1161/CIR.0000000000000603. ISSN 0009-7322.
  2. ↑ Shi, Guocheng; Zhu, Zhongqun; Chen, Jimei; Ou, Yanqiu; Hong, Haifa; Nie, Zhiqiang; Zhang, Haibo; Liu, Xiaoqing; Zheng, Jinghao; Sun, Qi; Liu, Jinfen; Chen, Huiwen; Zhuang, Jian (2017). "Total Anomalous Pulmonary Venous Connection". Circulation. 135 (1): 48–58. doi:10.1161/CIRCULATIONAHA.116.023889. ISSN 0009-7322.
  3. ↑ . doi:10.4103/2F0974-2069.157025. Missing or empty |title= (help)
  4. ↑ Attenhofer Jost, Christine H.; Connolly, Heidi M.; Dearani, Joseph A.; Edwards, William D.; Danielson, Gordon K. (2007). "Ebstein's Anomaly". Circulation. 115 (2): 277–285. doi:10.1161/CIRCULATIONAHA.106.619338. ISSN 0009-7322.
  5. ↑ Idrizi S, Milev I, Zafirovska P, Tosheski G, Zimbakov Z, Ampova-Sokolov V, Angjuseva T, Mitrev Z (September 2015). "Interventional Treatment of Pulmonary Valve Stenosis: A Single Center Experience". Open Access Maced J Med Sci. 3 (3): 408–12. doi:10.3889/oamjms.2015.089. PMC 4877828. PMID 27275259.
  6. ↑ Aykanat A, Yavuz T, Özalkaya E, Topçuoğlu S, Ovalı F, Karatekin G (January 2016). "Long-Term Prostaglandin E1 Infusion for Newborns with Critical Congenital Heart Disease". Pediatr Cardiol. 37 (1): 131–4. doi:10.1007/s00246-015-1251-0. PMID 26260095.
  7. ↑ Boucek DM, Qureshi AM, Goldstein BH, Petit CJ, Glatz AC (January 2019). "Blalock-Taussig shunt versus patent ductus arteriosus stent as first palliation for ductal-dependent pulmonary circulation lesions: A review of the literature". Congenit Heart Dis. 14 (1): 105–109. doi:10.1111/chd.12707. PMID 30811802.
  8. ↑ Vejlstrup, Niels; Sørensen, Keld; Mattsson, Eva; Thilén, Ulf; Kvidal, Per; Johansson, Bengt; Iversen, Kasper; Søndergaard, Lars; Dellborg, Mikael; Eriksson, Peter (2015). "Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark". Circulation. 132 (8): 633–638. doi:10.1161/CIRCULATIONAHA.114.010770. ISSN 0009-7322.
  9. ↑ Norwood WI, Jacobs ML (November 1993). "Fontan's procedure in two stages". Am. J. Surg. 166 (5): 548–51. doi:10.1016/s0002-9610(05)81151-1. PMID 8238751.
  10. ↑ d'Udekem, Y.; Iyengar, A. J.; Cochrane, A. D.; Grigg, L. E.; Ramsay, J. M.; Wheaton, G. R.; Penny, D. J.; Brizard, C. P. (2007). "The Fontan Procedure: Contemporary Techniques Have Improved Long-Term Outcomes". Circulation. 116 (11_suppl): I-157–I-164. doi:10.1161/CIRCULATIONAHA.106.676445. ISSN 0009-7322.
  11. ↑ . doi:10.1177/2F2150135118817765. Missing or empty |title= (help)
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